A 27-year-old woman developed a rapidly progressive left orbital tumor which extended into the cranial fossa. MRI revealed a heterogenous enhancing lesion confined to the left frontal bone and superior orbit. An excisional biopsy was performed and examination of the specimen showed findings diagnostic of a high-grade0020myxofibrosarcoma. Myxofibrosarcoma is a fibroblast-derived soft tissue neoplasm with up to a 60% local recurrence rate and metastasis may be associated with intermediate to high grade tumors. Complete excision with postoperative adjuvant radiation therapy with chemotherapy was performed.

Purpose To report a patient with a tumor-like corneal keloid. Methods Retrospective review of clinical features and histopathologic findings. Results A 68-year-old woman with a remote history of an orbital tumor treated with radiation developed a corneal tumor. A biopsy of the tumor showed fibrocellular tissue and her blind, painful eye was enucleated. Pathologic findings in the enucleated eye showed that the corneal mass was consistent with a large keloid. Conclusions A corneal keloid may clinically appear as a large corneal tumor.

Purpose To report a case of lichen planus in a patient with a history of herpes simplex virus keratitis. Methods Case report. Results A 60-year-old woman with chronic conjunctivitis and a history of herpes simplex virus keratitis was evaluated for irritation and a plaque on her right upper and lower eyelid palpebral conjunctivae. A surgical excision showed acanthosis and an underlying lichenoid infiltrate with a thickened basement membrane. Conclusion Lichen planus of the conjunctiva can be present in the absence of cicatrization in a patient with chronic irritation of the conjunctiva.

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. Herein, we describe the clinical and histologic spectrum of LCH of the orbit on the basis of five cases. One patient exhibited multifocal unisystem disease, the other four patients presented with a localized process. The typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunostaining. Transmission electron microscopy exhibited characteristic intracytoplasmic Birbeck granules. The different ophthalmic manifestations of LCH and treatment strategies are reviewed in the context of previously reported cases. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH has to be included in the differential diagnosis in tumors of the ocular adnexae, in particular in young children.

Purpose: To report the clinicopathologic features of three patients with CD30+ lymphoid proliferations of the eyelid. Design: Retrospective case series. Participants: Patients with cutaneous CD30+ lymphoproliferative lesions of the eyelid. Methods: Three patients with CD30+ non-mycosis fungoides T cell lymphoid infiltrates of the eyelid were identified. The histories, clinical findings, pathologic features including immunohistochemical staining, treatments and outcomes were reviewed and compared. Main Outcome Measures: Pathologic findings including immunohistochemical analysis. Results: The patients included an 81-year-old man, an 18-year-old man, and a 42-year-old woman, with CD30+ lymphoid proliferations of the eyelid and adjacent soft tissue. The first patient had an isolated crateriform eyelid lesion that was classified as lymphomatoid papulosis (LyP). The second patient had an isolated multinodular lesion of the eyelid that was classified as cutaneous anaplastic large cell lymphoma (cALCL). The third patient presented with eyelid edema with an underlying mass and was found to have widely disseminated anaplastic large cell lymphoma (ALCL). Diagnoses were dependent on clinical findings. Conclusions: CD30+ lymphoid proliferations represent a spectrum of conditions ranging from indolent LyP, to moderately aggressive cALCL, and to highly aggressive ALCL. Interpretation of the pathologic findings in CD30+ lymphoid proliferations is based in part on clinical findings.

OBJECTIVE: Blepharospasm is a type of dystonia where the diagnosis is often delayed because its varied clinical manifestations are not well recognized. The purpose of this study was to provide a comprehensive picture of its clinical features including presenting features, motor features, and non-motor features. METHODS: This was a two-part study. The first part involved a systematic literature review that summarized clinical features for 10,324 cases taken from 41 prior reports. The second part involved a summary of clinical features for 884 cases enrolled in a large multicenter cohort collected by the Dystonia Coalition investigators, along with an analysis of the factors that contribute to the spread of dystonia beyond the periocular region. RESULTS: For cases in the literature and the Dystonia Coalition, blepharospasm emerged in the 50s and was more frequent in women. Many presented with non-specific motor symptoms such as increased blinking (51.9%) or non-motor sensory features such as eye soreness or pain (38.7%), photophobia (35.5%), or dry eyes (10.7%). Non-motor psychiatric features were also common including anxiety disorders (34-40%) and depression (21-24%). Among cases presenting with blepharospasm in the Dystonia Coalition cohort, 61% experienced spread of dystonia to other regions, most commonly the oromandibular region and neck. Features associated with spread included severity of blepharospasm, family history of dystonia, depression, and anxiety. CONCLUSIONS: This study provides a comprehensive summary of motor and non-motor features of blepharospasm, along with novel insights into factors that may be responsible for its poor diagnostic recognition and natural history.

Six patients with mucinous eccrine carcinoma of the eyelid were identified in the case records of the L.F. Montgomery Laboratory. Five of these patients had undergone Mohs micrographic surgery after their initial diagnosis. Follow-up ranged from three months to eight years. Four patients with negative margins of excision had no evidence of recurrence, one patient was lost to follow-up and one patient with positive margins of excision had two recurrences after incomplete excision of the tumor. We recommend surgical excision of mucinous eccrine carcinoma of the eyelid with histologic monitoring of the surgical margins of resection.

Background The optimal initial local treatment for patients with Graves’ ophthalmopathy (GO) is not fully characterized. The purpose of this retrospective study is to describe the clinical outcomes of RT as initial local therapy for GO and define predictors of the need for post-RT salvage bony decompressive surgery. Methods 91 patients with active GO and without prior surgery were treated with RT as initial local therapy between 01/1999 and 12/2010, with a median follow-up period of 18.3 months (range 3.7 - 142 months). RT dose was 24 Gy in 12 fractions. 44 patients (48.4%) had prior use of steroids, with 31 (34.1%) being on steroids at the initiation of RT. The most common presenting symptoms were diplopia (79%), proptosis (71%) and soft tissue signs (62%). Results 84 patients (92.3%) experienced stabilization or improvement of GO symptoms. 58 patients (64%) experienced improvement in their symptoms. 19 patients (20.9%) underwent salvage post-RT bony decompressive surgery. Smoking status and total symptom score at 4 months were independent predictors of post-RT bony decompression with odds ratios of 3.23 (95% CI 1.03 – 10.2) and 1.59 (95% CI 1.06 – 2.4), respectively. Persistent objective vision loss at 4 months post-RT was the most important symptom type in predicting salvage decompression. Chronic dry eye occurred in 9 patients (9.9%) and cataracts developed in 4 patients (4.4%). Conclusions RT is effective and well tolerated as initial local therapy for active GO, with only 21% of patients requiring decompressive surgery post RT. Most patients experience stabilization or improvement of GO symptoms, but moderate to significant response occurs in the minority of patients. Smoking status and total symptom severity at 4 months, primarily persistent objective vision loss, are the primary determinants of the need for post-RT salvage bony decompression. Patients who smoke or present with predominantly vision loss symptoms should be advised as to their lower likelihood of symptomatic response to RT and their increased likelihood of requiring post-RT decompressive surgery.

Chondroid syringoma is a benign mixed tumor characterized by sweat gland elements in a cartilaginous stroma. This rare tumor accounts for only 0.01% of all primary skin tumors and occurs only rarely in the periorbital region. Chondroid syringomas are usually 0.5 to 3.0 cm in size, with increased risk of malignancy in tumors larger than 3.0 cm. Here we report a rare case of giant chondroid syringoma arising in the lower eyelid, characterized by an epithelial component in a cartilaginous stroma.